Aplastic crisis.
Red blood cell aplasia in pediatric patients who have chronic hemolysis is associated most frequently with B19 infection. Although this entity is usually recognized easily, other red cell hypoplastic anemias, such as TEC and Diamond-Blackfan anemia, must be considered as part of the differential diagnosis. Although usually a transient event, an aplastic crisis has the potential for significant morbidity. The patient usually can be supported through the episode without incident with the judicious use of erythrocyte transfusions. The recognition of the infectious nature of the event is important for an understanding of the clinical manifestation, course of illness, and need for isolation. Several questions remain unanswered regarding the pathogenesis and treatment of this disorder. Could the use of intravenous gamma-globulin prove effective in treating select cases of B19-induced red cell aplasia? Are there effective measures to prevent B19 infection in children at risk for significant morbidity from such infection? There is interest in development of a vaccine to B19, and children with hereditary hemolytic anemias represent a potential target group for its use. Are there other viruses that cause red cell aplasia, especially in the case of TEC? It is hoped that current research will provide the answers for more effective treatment and possible prevention of these aplastic crises.
Koch
W C
WC
Department of Pediatrics, Children's Medical Center, Medical College of Virginia, Richmond.
Massey
G V
GV
D000755:Anemia, Sickle Cell / Q000503:physiopathology* / Q000628:therapy; D002648:Child; D006801:Humans